Is pemphigus herpetiformis an entity?

Area of Science:

• Dermatology
• Immunodermatology
• Autoimmune Blistering Diseases

Background:

• Pemphigus herpetiformis is a rare autoimmune blistering disease.
• It can be initially misdiagnosed as dermatitis herpetiformis, IgA linear bullous dermatosis, or bullous pemphigoid.
• Understanding its distinct clinical, histological, and immunofluorescence features is crucial for accurate diagnosis and management.

Purpose of the Study:

• To characterize the clinical, histological, and immunofluorescence findings in patients with pemphigus herpetiformis.
• To evaluate the therapeutic responses to various treatment regimens.
• To analyze the disease course and potential evolution of skin lesions.

Main Methods:

• Clinical examination of 15 patients with pemphigus herpetiformis.
• Histopathological analysis of skin biopsies.
• Direct and indirect immunofluorescence studies using various substrates.
• Assessment of treatment efficacy with sulfones, prednisone, and cyclophosphamide.

Main Results:

• Histology revealed eosinophilic spongiosis or acantholysis with microabscesses.
• Direct immunofluorescence consistently showed intercellular IgG deposition.
• Indirect immunofluorescence was positive in 5/15 patients.
• Approximately 50% of patients responded to sulfones and prednisone; some required combined therapy with cyclophosphamide or higher prednisone doses.
• Nine patients maintained pemphigus herpetiformis features upon relapse, while others showed pemphigus seborrheicus-foliaceus type lesions.

Conclusions:

• Pemphigus herpetiformis exhibits characteristic intercellular IgG staining on direct immunofluorescence.
• Treatment often necessitates a combination of immunosuppressive agents.
• The disease may evolve into other pemphigus subtypes during relapses.